scholarly journals A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan

2020 ◽  
Author(s):  
Yuko Hamasaki ◽  
Riku Hamada ◽  
Masaki Muramatsu ◽  
Shinsuke Matsumoto ◽  
Kunihiko Aya ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Congenital Nephrotic Syndrome ◽  
Nationwide Survey ◽  
The Other ◽  
End Stage Kidney Disease ◽  
Cross Sectional ◽  
Infantile Nephrotic Syndrome ◽  
Finnish Type ◽  
End Stage

Abstract Background: Congenital nephrotic syndrome (CNS) and infantile nephrotic syndrome (INS) cause substantial morbidity and mortality. In Japan, there is a lack of knowledge regarding the characteristics of CNS and INS. This study aimed to clarify the characteristics of CNS and INS in Japan.Methods: This cross-sectional nationwide survey obtained data from 44 institutions in Japan managing 92 patients with CNS or INS by means of two survey questionnaires sent by postal mail. Patients aged <16 years by 1 April 2015, with a diagnosis of CNS or INS, were included in this study. The primary outcome was end-stage kidney disease.Results: A total of 83 patients with CNS or INS were analyzed. The most frequent disease type was non-Finnish (60.2%); 33 patients (39.8%) had Finnish type. Among those with non-Finnish-type disease, 26 had no syndrome and 24 had a syndrome, of which the most frequent was Denys–Drash syndrome (70.8%). Patients with non-Finnish-type disease with syndrome showed the earliest progression to end-stage kidney disease compared with the other two groups, whereas patients with non-Finnish-type disease without syndrome progressed more slowly compared with the other two groups. In the Finnish-type group, the disease was diagnosed the earliest; a large placenta was reported more frequently; genetic testing was more frequently performed (93.8%); mental retardation was the most frequent extra-renal symptom (21.2%); and thrombosis and infection were more frequent compared with the other groups. Patients with non-Finnish-type disease with syndrome had a higher frequency of positive extra-renal symptoms (79.2%), the most common being urogenital symptoms (54.2%). Treatment with steroids and immunosuppressants was more frequent among patients with non-Finnish-type disease without syndrome. Two patients with non-Finnish-type disease without syndrome achieved complete remission. In all groups, unilateral nephrectomy was performed more often than bilateral nephrectomy and peritoneal dialysis was the most common renal replacement therapy.Conclusions: The present epidemiological survey sheds light on the characteristics of children with CNS and INS in Japan. A high proportion of patients underwent genetic examination, and patient management was in accord with current treatment recommendations and practices.Trial registration: Not applicable.

scholarly journals A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan

2020 ◽  
Author(s):  
Yuko Hamasaki ◽  
Riku Hamada ◽  
Masaki Muramatsu ◽  
Shinsuke Matsumoto ◽  
Kunihiko Aya ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Congenital Nephrotic Syndrome ◽  
Nationwide Survey ◽  
The Other ◽  
End Stage Kidney Disease ◽  
Cross Sectional ◽  
Infantile Nephrotic Syndrome ◽  
Finnish Type ◽  
End Stage

Abstract Background: Congenital nephrotic syndrome (CNS) and infantile nephrotic syndrome (INS) cause substantial morbidity and mortality. In Japan, there is a lack of knowledge regarding the characteristics of CNS and INS. This study aimed to clarify the characteristics of CNS and INS in Japan.Methods: This cross-sectional nationwide survey obtained data from 44 institutions in Japan managing 92 patients with CNS or INS, by means of two survey questionnaires sent by postal mail. Patients aged <16 years by 1 April 2015, with a diagnosis of CNS or INS, were included in this study. The primary outcome was end-stage kidney disease.Results: A total of 83 patients with CNS or INS were analyzed. The most frequent disease type was non-Finnish (60.2%); 33 patients (39.8%) had Finnish type. Among those with non-Finnish-type disease, 26 had no syndrome and 24 had a syndrome, of which the most frequent was Denys–Drash syndrome (70.8%). Patients with non-Finnish-type disease with syndrome showed the earliest progression to end-stage kidney disease compared with the other two groups, whereas patients with non-Finnish-type disease without syndrome progressed more slowly compared with the other two groups. In the Finnish-type group, the disease was diagnosed the earliest; a large placenta was reported more frequently; genetic testing was more frequently performed (93.8%); mental retardation was the most frequent extra-renal symptom (21.2%); and thrombosis and infection were more frequent compared with the other groups. Patients with non-Finnish-type disease with syndrome had a higher frequency of positive extra-renal symptoms (79.2%), the most common being urogenital symptoms (54.2%). Treatment with steroids and immunosuppressants was more frequent among patients with non-Finnish-type disease without syndrome. Two patients with non-Finnish-type disease without syndrome achieved complete remission. In all groups, unilateral nephrectomy was performed more often than bilateral nephrectomy and peritoneal dialysis was the most common renal replacement therapy.Conclusions: The present epidemiological survey sheds light on the characteristics of children with CNS and INS in Japan. A high proportion of patients underwent genetic examination, and patient management was in accord with current treatment recommendations and practices.Trial registration: Not applicable.

scholarly journals Non-medical barriers reported by nephrologists when providing renal replacement therapy or comprehensive conservative management to end-stage kidney disease patients: a systematic review

2020 ◽  
Author(s):  
Rianne W de Jong ◽  
Vianda S Stel ◽  
James G Heaf ◽  
Mark Murphy ◽  
Ziad A Massy ◽  
...  
Keyword(s):  
Systematic Review ◽  
Renal Replacement Therapy ◽  
Kidney Disease ◽  
Focus Groups ◽  
Replacement Therapy ◽  
Conservative Management ◽  
End Stage Kidney Disease ◽  
Cross Sectional ◽  
Financial Barriers ◽  
End Stage

Abstract Background Large international differences exist in access to renal replacement therapy (RRT) modalities and comprehensive conservative management (CCM) for patients with end-stage kidney disease (ESKD), suggesting that some patients are not receiving the most appropriate treatment. Previous studies mainly focused on barriers reported by patients or medical barriers (e.g. comorbidities) reported by nephrologists. An overview of the non-medical barriers reported by nephrologists when providing the most appropriate form of RRT (other than conventional in-centre haemodialysis) or CCM is lacking. Methods We searched in EMBASE and PubMed for original articles with a cross-sectional design (surveys, interviews or focus groups) published between January 2010 and September 2018. We included studies in which nephrologists reported barriers when providing RRT or CCM to adult patients with ESKD. We used the barriers and facilitators survey by Peters et al. [Ruimte Voor Verandering? Knelpunten en Mogelijkheden Voor Verbeteringen in de Patiëntenzorg. Nijmegen: Afdeling Kwaliteit van zorg (WOK), 2003] as preliminary framework to create our own model and performed meta-ethnographic analysis of non-medical barriers in text, tables and figures. Results Of the 5973 articles screened, 16 articles were included using surveys (n = 10), interviews (n = 5) and focus groups (n = 1). We categorized the barriers into three levels: patient level (e.g. attitude, role perception, motivation, knowledge and socio-cultural background), level of the healthcare professional (e.g. fears and concerns, working style, communication skills) and level of the healthcare system (e.g. financial barriers, supportive staff and practice organization). Conclusions Our systematic review has identified a number of modifiable, non-medical barriers that could be targeted by, for example, education and optimizing financing structure to improve access to RRT modalities and CCM.

scholarly journals Study of Cutaneous Manifestations in End Stage Kidney Disease Undergoing Hemodialysis

2020 ◽  
Vol 18 (1) ◽  
pp. 37-43
Author(s):  
Abhishek Maskey ◽  
Ajay Kumar ◽  
Roshan Shrestha
Keyword(s):  
Kidney Disease ◽  
Maintenance Hemodialysis ◽  
Cholestatic Liver Disease ◽  
End Stage Kidney Disease ◽  
Major Step ◽  
Cross Sectional ◽  
Dialysis Unit ◽  
Cutaneous Manifestations ◽  
The Mean ◽  
End Stage

Introduction: The prevalence of cutaneous manifestations in hemodialysis patients is increasing. Objectives: The aim of this study was to determine the prevalence and pattern of various cutaneous manifestations in patients undergoing maintenance hemodialysis. Material and Methods: A hospital based cross sectional study was conducted in patient undergoing maintenance hemodialysis at least for three months in dialysis unit of Manipal Teaching Hospital Pokhara, Nepal during the period from August 2018 to January 2019. A demographic questionnaire and a checklist about cutaneous disorders were used for data collection. Patients with cholestatic liver disease or acute hepatitis, active infection, active malignancy, patient with acute kidney injury, patient undergoing peritoneal dialysis and renal transplant recipient were excluded from study. Results: Total 80 patients undergoing maintenance hemodialysis were included. Among them, 52 (65%) patients were male. The mean age of study population was 51.95±14.96 years. The mean duration of dialysis was 40.28±11.09 months. The most common cause of end stage kidney disease was diabetic nephropathy. The most common cutaneous manifestations were pigmentation (82.5%), nail changes (75%), xerosis (70%) and pruritis (50%). Conclusions: The results of this study revealed that patients on hemodialysis were associated with multiple cutaneous symptoms, the most prevalent of which were pigmentation and nail disorders. Therefore, early diagnosis of these problems is a major step in improving the quality of life in these patients.

scholarly journals Primary Nephrotic Syndrome and Risks of End-Stage Kidney Disease, Cardiovascular Events, and Death: The Kaiser Permanente Nephrotic Syndrome Study

2021 ◽  
pp. ASN.2020111583
Author(s):  
Alan Go ◽  
Thida Tan ◽  
Glenn Chertow ◽  
Juan Ordonez ◽  
Dongjie Fan ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Membranous Nephropathy ◽  
Minimal Change Disease ◽  
Cardiovascular Outcomes ◽  
Minimal Change ◽  
End Stage Kidney Disease ◽  
Kaiser Permanente ◽  
Primary Nephrotic Syndrome ◽  
End Stage

Background Few population-based data exist about adults with primary nephrotic syndrome. Methods To evaluate kidney, cardiovascular, and mortality outcomes in adults with primary nephrotic syndrome, we identified adults within an integrated health care delivery system (Kaiser Permanente Northern California) with nephrotic-range proteinuria or diagnosed nephrotic syndrome between 1996-2012. Nephrologists reviewed medical records for clinical presentation, laboratory findings, and biopsy results to confirm primary nephrotic syndrome and assigned etiology. We identified a 1:100 time-matched cohort of adults without diabetes, diagnosed nephrotic syndrome, or proteinuria as controls to compare rates of end-stage kidney disease (ESKD), cardiovascular outcomes, and death through 2014, using multivariable Cox regression. Results We confirmed 907 cases of primary nephrotic syndrome (655 definite and 252 presumed cases of focal segmental glomerulosclerosis [FSGS, 40%], membranous nephropathy [40%], and minimal change disease [20%]). Mean age was 49 years; 43% were women. Adults with primary nephrotic syndrome had higher adjusted rates of ESKD (adjusted hazard ratio [aHR], 19.63; 95% confidence interval [95% CI], 12.76 to 30.20), acute coronary syndrome (aHR 2.58; 95% CI, 1.89 to 3.52), heart failure (aHR 3.01; 95%CI, 2.16 to 4.19), ischemic stroke (aHR 1.80; 95%CI,1.06 to 3.05), venous thromboembolism (aHR 2.56; 95%CI, 1.35 to 4.85), and death (aHR 1.34; 95%CI, 1.09 to 1.64) versus controls. Excess ESKD risk was significantly higher for FSGS and membranous nephropathy than for presumed minimal change disease. The three etiologies of primary nephrotic syndrome did not differ significantly in terms of cardiovascular outcomes and death. Conclusions Adults with primary nephrotic syndrome experience higher adjusted rates of ESKD, cardiovascular outcomes, and death, with significant variation by underlying etiology in risk for developing ESKD.

scholarly journals SO045A NATIONWIDE SURVEY ON RADIATION RISK OF CANCER FROM MEDICAL IMAGING IN END STAGE KIDNEY DISEASE AND IN TRANSPLANT PATIENTS

2016 ◽  
Vol 31 (suppl_1) ◽  
pp. i20-i20
Author(s):  
Marco Brambilla ◽  
Domenico Lizio ◽  
Andreana De Mauri ◽  
Carmela Marino ◽  
Carmine Zoccali ◽  
...  
Keyword(s):  
Medical Imaging ◽  
Kidney Disease ◽  
Radiation Risk ◽  
Nationwide Survey ◽  
End Stage Kidney Disease ◽  
Transplant Patients ◽  
End Stage ◽  
Risk Of Cancer

scholarly journals Status of care for end stage kidney disease in countries and regions worldwide: international cross sectional survey

BMJ ◽  
2019 ◽  
pp. l5873 ◽  
Author(s):  
Aminu K Bello ◽  
Adeera Levin ◽  
Meaghan Lunney ◽  
Mohamed A Osman ◽  
Feng Ye ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Kidney Disease ◽  
Replacement Therapy ◽  
Low Income ◽  
Care Delivery ◽  
End Stage Kidney Disease ◽  
Cross Sectional Survey ◽  
Cross Sectional ◽  
Kidney Replacement Therapy ◽  
End Stage

Abstract Objective To determine the global capacity (availability, accessibility, quality, and affordability) to deliver kidney replacement therapy (dialysis and transplantation) and conservative kidney management. Design International cross sectional survey. Setting International Society of Nephrology (ISN) survey of 182 countries from July to September 2018. Participants Key stakeholders identified by ISN’s national and regional leaders. Main outcome measures Markers of national capacity to deliver core components of kidney replacement therapy and conservative kidney management. Results Responses were received from 160 (87.9%) of 182 countries, comprising 97.8% (7338.5 million of 7501.3 million) of the world’s population. A wide variation was found in capacity and structures for kidney replacement therapy and conservative kidney management—namely, funding mechanisms, health workforce, service delivery, and available technologies. Information on the prevalence of treated end stage kidney disease was available in 91 (42%) of 218 countries worldwide. Estimates varied more than 800-fold from 4 to 3392 per million population. Rwanda was the only low income country to report data on the prevalence of treated disease; 5 (<10%) of 53 African countries reported these data. Of 159 countries, 102 (64%) provided public funding for kidney replacement therapy. Sixty eight (43%) of 159 countries charged no fees at the point of care delivery and 34 (21%) made some charge. Haemodialysis was reported as available in 156 (100%) of 156 countries, peritoneal dialysis in 119 (76%) of 156 countries, and kidney transplantation in 114 (74%) of 155 countries. Dialysis and kidney transplantation were available to more than 50% of patients in only 108 (70%) and 45 (29%) of 154 countries that offered these services, respectively. Conservative kidney management was available in 124 (81%) of 154 countries. Worldwide, the median number of nephrologists was 9.96 per million population, which varied with income level. Conclusions These comprehensive data show the capacity of countries (including low income countries) to provide optimal care for patients with end stage kidney disease. They demonstrate substantial variability in the burden of such disease and capacity for kidney replacement therapy and conservative kidney management, which have implications for policy.

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